We report a case of IgG/IgA pemphigus presenting as pemphigus foliaceus following diagnosis and treatment of classic IgG‐mediated pemphigus vulgaris. The dual presentation of IgG and IgA positivity on direct immunofluorescence (DIF) constitutes a rare form of pemphigus with a wide variety of clinicopathologic manifestations. The progression of pemphigus vulgaris is commonly because of epitope spreading. However, the patient's disease was partially refractory to rituximab and showed a change in the DIF with dual staining for IgG and IgA. This indicates that class‐switching may have occurred with epitope spreading or that there was autoreactive IgA at the onset of disease below the threshold of detection by DIF. Our case indicates that in progressive autoimmune disease refractory to treatment, re‐evaluation of the patient for antibody isotypes absent on initial diagnosis may offer key information in better identifying the cause of progression as well as in directing the necessary treatment.
infection is usually asymptomatic but can result in a hyperinfection syndrome, most commonly triggered by acquired or iatrogenic immunosuppression. Here, we present a case of a man aged 60 years originally from a strongyloides endemic area with a medical history of alcohol abuse who presents with strongyloides hyperinfection syndrome (SHS) complicated by partial small bowel obstruction, pulmonary haemorrhage, large bandemia without eosinophilia and cardiac arrest resulting in death. This case is notable for the presence of bandemia and absence of eosinophilia, lack of historical risk factors for hyperinfection, specifically corticosteroid immunosuppressants, and dramatic decline in clinical status which ultimately resulted in the patient's death. Clinicians should suspect SHS in immunocompetent patients who are from an endemic area and who have persistent gastrointestinal and/or pulmonary manifestations in the absence of a clear cause.
Fibrous dysplasia is a dysplastic disorder of bone caused by missense mutations of the GNAS1 gene. Fibrocartilaginous dysplasia (also known as fibrochondrodysplasia or fibrous dysplasia with extensive cartilaginous differentiation) is a rare variant of fibrous dysplasia with extensive cartilaginous differentiation that shows a predilection for the lower extremities and can affect one or multiple bones. Although benign, this entity causes progressively expansile lesions that can lead to deformity and ultimately pathologic fracture. On imaging, this entity can show features consistent with a variety of benign and malignant cartilage-forming tumors. We present the case of a 16-year-old male patient who presented with clinical history of Ollier disease and multiple presumed enchondromas. He underwent below-the-knee amputation for progressive tibial deformity, pathologic fracture, and pain. Microscopic examination of multiple lower-extremity bones revealed a fibro-osseous lesion with massive cartilaginous differentiation consistent with fibrocartilaginous dysplasia.
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